RESUMO
El tumor neuroectodérmico maligno del tracto gastrointestinal es una neoplasia rara con pocos casos reportados en la literatura, especialmente en América Latina. Descrito por primera vez en 2003, se trata de una entidad sin tratamiento estandarizado y de pobre pronóstico. Se presenta el caso de una paciente de 22 años de edad que acude a la consulta por dolor abdominal, anemia y masa abdominal palpable. Luego de estudios pertinentes se decide la conducta resectiva y el posterior tratamiento oncológico. (AU)
Malignant gastrointestinal neuroectodermal tumor (GNET), formerly known as clear cell sarcoma of the gastrointestinal tract, is an extremely rare tumor of mesenchymal origin, which presents great microscopic and molecular similarity to clear cell sarcoma found in other parts of the body, such as tendons and aponeurosis. It is characterized by its rapid evolution, high recurrence rate and frequent diagnosis as metastatic disease.1,2 (AU)
Assuntos
Humanos , Feminino , Adulto Jovem , Sarcoma de Células Claras/patologia , Tumores Neuroectodérmicos/patologia , Neoplasias Gastrointestinais/diagnóstico , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Imuno-Histoquímica , Proteínas S100/análise , Neoplasias Gastrointestinais/cirurgia , Íleo/cirurgiaRESUMO
We present the autopsy findings and differential diagnosis in a 42year old male who presented with fever and rapidly progressive respiratory symptoms like breathlessness, nonproductive cough and right sided chest pain. Initial imaging workup done at our hospital revealed a large unilateral tumor with tracheal shift. While being evaluated patient developed facial puffiness, tachypnea suggestive of superior vena cava obstruction. Antemortem biopsy of lung mass was attempted twice and that suggested malignant lesion. Unfortunately, the individual had a rapid downhill course following admission. Post mortem examination was conducted that on opening the thoracic cavity revealed total replacement of right lung tissue by a necrotic growth which was deeply adherent to the rib cage. The contralateral lung as well as all other visceral organs were unremarkable grossly. Histopathology confirmed primary Ewing sarcoma of the lung. We hereby, report a rare case of primary lung Ewing sarcoma diagnosed at autopsy.
Assuntos
Humanos , Masculino , Adulto , Sarcoma de Ewing/patologia , Neoplasias Ósseas/patologia , Tumores Neuroectodérmicos/patologia , Neoplasias Pulmonares/patologia , Autopsia , Evolução Fatal , Diagnóstico DiferencialRESUMO
Se presenta un lactante menor de 45 días de vida, que consultó a nuestro servicio por múltiples lesiones nodulares y tumorales azuladas, de aparición rápida, de 15 días de evolución, que llevó a elaborar los diferentes diagnósticos diferenciales de las entidades que causan el síndrome de blueberry muffin baby. Los estudios confirmaron el diagnóstico de neuroblastoma.
We report an infant under 45 days old, who presented to our department with multiple bluish nodular lesions and tumors of rapid onset and 15 days of evolution, which led to raise the differential diagnoses of the entities causing the blueberry muffin baby syndrome. The studies confirmed the diagnosis of neuroblastoma.
Assuntos
Humanos , Masculino , Lactente , Neuroblastoma , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/tratamento farmacológico , Tumores Neuroectodérmicos/diagnóstico , Tumores Neuroectodérmicos/patologia , Tumores Neuroectodérmicos/tratamento farmacológicoRESUMO
Se presenta un caso de meduloepitelioma maligno del adulto de la variedad no teratoide. La neoplasia se originó en el globo ocular, invadió a la órbita y secundariamente a las fosas nasales. Se discute la biología e histogénesis de esta rara neoplasia y la clasificación general de los tumores neuroectodérmicos de las fosas nasales. Se hace énfasis en la importancia del diagnóstico diferencial en especial por la confusión diagnóstica histopatológica inicial